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Flashcards in CF Deck (7)

What is the mutation that causes of CF?
What is the cause of this mutation?

deletion of delta508 in CFTR gene on chrom 7q

defective CFTR Cl- channel


How does a baby with CF present?

-salty skin
-meconium illius (no abnormalities with GI tract, poop just gets stuck due to super viscous pancreatic secretions)
-failure to thrive


What causes all the problems in CF?

thick mucous secretions createes plugs in lungs, pancreas, and liver


What problems do CF pts have?

chronic bronhcitis --> bronchiectasis
pancreatic insufficiency --> FATTY REPLACEMENT
billiary cirrhosis
fat soluble vitamin def
infertility in men


How is CF diagnosed?

high Cl- conc in sweat test


What pneumonia is assc with CF?

pneudomonas and s.aureus


What is the treatment for CF?

N-Acetylcysteine loosens plugs by cleaving disulfide bonds in mucous glycoproteins